Pediatr Nephrol (2012) 27:1547–1550 DOI 10.1007/s00467-012-2172-2
ORIGINAL ARTICLE
Nephrotic syndrome in Kawasaki disease: a report of three cases Pauline Krug & Olivia Boyer & Eve Balzamo & Daniel Sidi & Agnès Lehnert & Patrick Niaudet
Received: 5 February 2012 / Revised: 25 March 2012 / Accepted: 27 March 2012 / Published online: 24 April 2012 # IPNA 2012
Abstract Background Renal manifestations are rare in Kawasaki disease (KD). Acute renal failure with tubular necrosis, tubulointerstitial nephritis and renovascular hypertension have been reported in KD, but only one case of a patient with KD associated with nephrotic syndrome (NS) has been reported to date, with the patient improving on steroid therapy but dying from coronary aneurysm. Methods We report the cases of three children, aged 4, 4.5 and 8 years, respectively, who presented with typical KD symptoms (high fever, diffuse maculopapular rash, conjunctivitis, peripheral oedema, cervical adenopathies and high C reactive protein levels) and developed NS. Results Patient 1 had a haemodynamic shock due to cardiac dysfunction and transient renal failure. Ten days later, he developed a NS which spontaneously disappeared 1 week later. Patient 2 had a NS on admission with normal plasma creatinine and no haematuria. Proteinuria disappeared within 10 days. Patient 3 developed NS 5 days after onset with a moderate increase in plasma creatinine. Proteinuria disappeared within 2 weeks. All three patients were treated with intravenous immunoglobulins, antibiotic therapy and aspirin, but none of them received steroid therapy. To date, all three patients have maintained long-term remission. P. Krug : O. Boyer : E. Balzamo : P. Niaudet (*) Pediatric Nephrology, Necker Hospital, Paris, France e-mail:
[email protected] D. Sidi Pediatric Cardiology, Necker Hospital, Paris, France A. Lehnert Pediatrics, Montargis Hospital, Montargis, France
Conclusions In conclusion, proteinuria with NS may develop during the acute phase of KD with persistent remission occurring without steroid therapy. Keywords Kawasaki disease . Nephrotic syndrome . Proteinuria . Parvovirus B19
Introduction Kawasaki disease (KD) is a childhood acute inflammatory disease. The diagnosis is based on the following clinical criteria: fever, bilateral non-exudative conjunctivitis, oral mucosa changes, rash, lymphadenopathies and oedema of the hands and feet. A fever of at least 5 days duration, the presence of four of the symptoms just described and the lack of another known aetiology are sufficient for the diagnosis [1]. Desquamation occurring 5 days after onset and a late thrombocytosis provide retrospective confirmation of the diagnosis. Other classical features associated with—but not included in—the clinical diagnosis criteria are erythema and induration at the sites of BCG immunisations, arthritis, uveitis, aseptic meningitis, coronary artery abnormalities visible on echocardiograms, hydrocholecyst on abdominal ultrasonography images and sterile pyuria [2]. Blood samples show signs of inflammatory syndrome, and thrombocytosis occurs at the end of the disease. The main clinical concern is the cardiovascular complications, hence the importance of diagnosis and early treatment. KD has been reported in all ethnic groups, and although it is more frequent in children aged less than 5 years, it has been described in the entire paediatric age range [3]. The cause of KD remains unknown although an infectious agent has been suspected based on seasonal peak frequencies and a clinical presentation suggestive of adenovirus or streptococcal
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infection. However, no aetiological agent has yet been identified. A second hypothesis is that of an immune response to a bacterial superantigen. The physiopathology of KD also remains unclear. T cells, monocytes and macrophages are implicated in the inflammatory process, with a high secretion of proinflammatory cytokines and chemokines during the acute phase of the disease; an immunoglobulin A (IgA) immune response is also probably implicated [3, 4]. In addition, a genetic susceptibility is suspected. Renal involvement is rare, usually consisting of sterile pyuria. Proteinuria of tubular and glomerular origin may be observed but is rarely associated with full-blown nephrotic syndrome (NS), characterized by proteinuria of >50 mg/kg/day and hypoalbuminaemia of 50 mg/kg/day) without renal failure or haematuria. The chest X-ray was normal, and urine and blood cultures were sterile. The results for the MNI-test and mycoplasma and human immunodeficiency virus (HIV) serology analyses were negative; the test for ASLO (anti-streptolysin O antibodies) was positive (300 units; normal
